Dandy–Walker syndrome

Abstract

DANDY–WALKER SYNDROME (DWS) is a congenital malformation of neural tube closure characterized by a localized defect in the differentiation of the hindbrain and it consists of three major abnormalities: (i) complete or partial agenesis of the cerebellar vermis (mainly its inferior portion); (ii) posterior fossa cyst (strictly speaking, a cystic dilatation of the fourth ventricle); and (iii) an enlarged posterior fossa with upward displacement of the tentorium, lateral sinuses, and torcular (Table 1). The triad is commonly associated with hydrocephalus, but this condition should be considered a very common complication (occurring in more than 80% of cases) and not part of the malformation.

DANDY–WALKER SYNDROME (DWS) is a congenital malformation of neural tube closure characterized by a localized defect in the differentiation of the hindbrain and it consists of three major abnormalities: (i) complete or partial agenesis of the cerebellar vermis (mainly its inferior portion); (ii) posterior fossa cyst (strictly speaking, a cystic dilatation of the fourth ventricle); and (iii) an enlarged posterior fossa with upward displacement of the tentorium, lateral sinuses, and torcular (Table 1). The triad is commonly associated with hydrocephalus, but this condition should be considered a very common complication (occurring in more than 80% of cases) and not part of the malformation.

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